Neurology

Neurology

Charcot giving a lecture on hysteria

Shamans and medicine men have poked and prodded the brain since the Stone age, with trepanation being the brainchild (forgiving the pun) of the Incas. Their handiwork in the form of golfball-sized holes in the cranial vault is a testimony to their surgical skills, the sangfroid of their patients, and/or the efficacy of their treatment for infections, given that by the 1400s, up to 90% of those treated actually survived. Across the ocean the field evolved less invasively as a medical art with the latter half of the 19th century and the early 20th century marking the so-called Golden Age of Neurology and the zenith of the European fathers of neurology. The gods of the era included Magendie (1783-1855), Romberg (1795-1873), Duchenne (1806-1875), Broca (1824-1880), Charcot (1825-1893, top image), Hughlings Jackson (1835-1911), Gowers (1845-1915), Pavlov (1849-1936), Binswanger (1852-1929), and Brodmann (1868-1918), just to mention a few of the superstars.

The Golden Age saw the development of tools and tests which are still an integral part of the physical and functional examination of neurologic patients: the tendon reflex hammer, tuning fork and ophthalmoscope, lumbar punctures and diagnostic radiology. As clinical neurology matured in the 20th century, other tools came online, including EEG (electroencephalography) in the 1930s, cerebral angiography in the 1950s, CT imaging in the 1970s, functional MRI in the 1990s. More recently, CT or MRI scans, which map the anatomy of the brain, can be performed in tandem with positron emission tomography (PET), which provides functional information of the brain, resulting in more precise information as PET-CT and PET-MRI. This is accomplished without the need for an integrated PET-CT or PET-MRI scanner, using a so-called N-localizer.

Caption from bottom image (edited) An elderly male patient presented with a first epileptic seizure.

Exciting as are the diagnostic tools now at neurologists’ disposal, what’s on the horizon for neurology is even more so. Still ripening on the vine, is a bumper crop of genes from the Human Genome Project (finished in 2003*), defects of which have been linked to a plethora of diseases. Many neurologic disorders–e.g., amyotrophic lateral sclerosis (ALS)†, Charcot-Tooth-Marie disease, and non-syndromic deafness, were once regarded as single entities. It is now known that whilst many hereditary conditions share the same clinical picture, they are usually caused by different genes. As an example, ALS1 is caused by a defect in SOD1—the gene that encodes superoxide dismutase 1; ALS9 is caused by a defect of ANG—the gene that encodes angiogenin, a potent mediator of normal and neoplastic angiogenesis; and ALS21 is caused by a defect of MATR3—the gene that encodes a nuclear matrix protein thought to stabilize certain mRNA species. This means that gene therapy, when it transitions from the research bench to the bedside…and it will, will respond to manipulation of the defective gene, and only that gene. These are exciting times.

*It was “roughed out” in 2003, more work is ongoing. †Popularly known in the USA as Lou Gehrig (1903-1941) disease, after the New York Yankees player who succumbed of the disease in his prime

Before leaving this section, I’d be remiss if I didn’t mention the training required of a neurologist (5 to 8 years after medical school), areas of practice (pain management, sleep disorders, audiology and rehabilitation medicine) and the so-called meat-and-potatoes conditions (stroke, dementia, epilepsy, migraine, neuromuscular disease and neuropathies).